Pulmozyme in Cystic Fibrosis With Sinusitis

The study will be a prospective enrollment of patients with CF and rhinosinusitis in a
double-blind crossover study with nasal nebulized DNASE versus saline. Nebulization will be
accomplished with PARI SinuStar nasal aerosol system nebulizer set powered by an air
compressor. This will be a pilot study with each subject serving as their own control. Each
arm of the study will be 3 months and randomization to active drug versus placebo will be
generated without the knowledge of subject or researchers. A health care provider not
involved in the care of the subject will be responsible for medication distribution and the
randomization scheme. A daily diary will collect data for analysis. Visits will be arranged
every 4 weeks at which time drug will be dispensed, adherence to daily diary and compliance
to medication will be assessed. At each visit the SF-12, SNOT-20, Epworth Sleepiness Scale,
Rhinitis Severity Scale, Nocturnal Rhinoconjunctivitis Quality of Life Specific
Questionnaire (NRQLQ), Cystic Fibrosis Questionnaire-Revised, and Rhinosinusitis Quality of
Life Survey will be utilized for acquisition of data (17-23). In addition, a Physician
Global Assessment and a self administered patient rhinosinusitis VAS score (0-100 with 100
full recovery and 0 no effect) will be completed at each visit. To obtain objective data
patients will have nasal endoscopic assessment at 4 different time points and nasal polyps
and sinusitis rated on a scale as defined by Lund and Kennedy in the Ann Otol Laryngol
1995;104 (suppl 167):17-21. (24) Serial sinus CT scans will be avoided to decrease
irradiation risk.

Subjects: A total of 12 patients will be enrolled in this pilot study, and recruitment will
be through the Cystic Fibrosis Center at Penn State University, after IRB approval. All
subjects must be over the age of 15 years (the age the investigators consider to be
necessary to understand the consent and questionnaires required for the study), be of either
gender and of any ethnicity. Subjects will be able to continue all presently used nasal and
respiratory medications as long as the present therapeutic regimen has been used for one
month prior to enrollment and these therapies have been used at a stable dose, method of
distribution and without adverse events

Inclusion Criteria:

1. Patients with CF, age >15yo

2. Chronic sinusitis with symptoms

3. VAS score of at least 60 (0 to 100) reflecting symptoms associated with
rhinosinusitis

4. CT scan demonstrating non-complicated sinusitis (defined as rhinosinusitis without
orbit perforation, osteomyelitis, peri-sinus cellulitis, or meningitis)

Exclusion Criteria:

1. No evidence of sinusitis

2. VAS score for rhinosinusitis less than 60 on a scale of 0 to 100

3. Complicated sinusitis (defined as orbit perforation, osteomyelitis, peri-sinus
cellulitis, meningitis complicating sinusitis)

4. Prior adverse events or allergy to DNASE

5. Unwilling to sign an approved IRB consent

6. Immediate indication for sinus surgery

7. Inability to adhere to therapy and understand and to complete questionnaires.

8. Being pregnant will exclude a subject from participating and the subjects will be
requested to take birth control methods if actively engaging in sex. Further more,
subjects will be requested to inform the investigators if they become pregnant.
Pregnancy test will be performed at screening.

9. Active nursing of an infant will be an exclusion.